We present 31 patients with cystine urolithiasis referred to our extracorporeal shock wave lithotripsy (ESWL) center since 1985. This group differed statistically from the other 3000 patients treated by us for urolithiasis with regard to age, stone burden, number of previous operations, and known duration of urolithiasis. The average known duration in those with cystine stones was 15 years. Of 26 patients with such a history, 22 had an average of 2 operations each. At referral, 14 had bilateral stones and among the others there were 5 nonfunctioning kidneys. Of 26 referred with a diagnosis of cystine nephrolithiasis, only 11 were being treated medically. At presentation, 31 had no stones and 1 was being treated medically. In the other 29 patients, 44 renal-ureteral units had urolithiasis. During follow-up 2 ureteral stones were discharged spontaneously and the other units were treated by ESWL, surgery and/or ureteroscopy, and percutaneous irrigation. 36/42 units were free of significant stone residue, but in 4 there were large gravel remains; 1 kidney was removed primarily and 1 kidney lost its function following prolonged obstruction after ESWL. During follow-up, of 11 patients unable to tolerate D-penicillamine and treated by urinary alkalinization alone, 3 have already formed new stones.

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